Download Rheumatic heart disease

Rheumaticheartdisease
DigitalPathologyCollection
Case142010
Ref.II:iv:2&II:iv:5
UniversityofCapeTown
Case1
Rheumaticvegetationsonmitralvalve
UniversityofCapeTown
Pathology:
Acuterheumaticendocarditis
• Thisheartlesionwasan
autopsyfindinginagirlof16
years.
• Thereisarowofsmall,firm,
greyishvegetationsalong
thecontactlineofthemitral
valvecusps.Inplacesthe
vegetationshavefusedto
formacontinuousridge.
UniversityofCapeTown
• Thevegetationshave
occurredatasiteofminor
trauma,wherethevalve
cuspscomeintocontact
eachtimethevalvecloses
i.e.onthecontactline(not
thefreeedgeofthevalve).
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• Therheumaticvegetationsorverrucaearisedueto
inflammatoryerosionofthevalvesurface,followedbyplatelet
andfibrinprecipitation.
• Theyarethecardinalfeatureofrheumaticendocarditis.
• Butendocarditisisjustonecomponentofthepancarditisthat
occursinacuterheumaticfever,i.e.thereareinflammatory
changesinallthreelayersoftheheart,endocarditis,
myocarditisandpericarditis.
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Anatomy
• Asidefromthevisiblerheumaticvegetations,theanatomyof
theheartandthemitralvalveisnormalatthisstage.
• Thehearthasbeenopenedinaverticalplanethroughtheleft
atriumandventricle.
• Themitralvalveringhasbeenopenedandflattenedout
horizontally.
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Leftatrium
Mitralvalve
LV
LV
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• Inthenextslide,noteinparticularthenormaldelicate
chordaetendineaeandthepapillarymuscles.
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Chordaetendineae
Papillarymuscles
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Chronicrheumaticheartdisease
• Inchronicrheumaticheartdisease(RHD)theinflammatory
lesionsintheheartundergoorganisationandfibrosis.
• Recurrentepisodesofrheumaticfevercausecumulativedamage.
• Inparticular,thevalvesbecomescarredanddistorted,affecting
theirfunction.
• Clinicalsignsofthevalvularandothercardiacdamagemaytake
yearsordecadestoappear.
• ThemitralvalveisalmostalwaysaffectedbyRHD,withor
withoutaorticvalveabnormality.Morerarelythereareright
sidedvalveabnormalities.
(Thinkaboutwhytheleftsidedvalvesare
moresusceptibletodamage.)
UniversityofCapeTown
UniversityofCapeTown
Mitralstenosis
• 99%ofcasesofmitralstenosisareattributabletoRHD.
• Inasignificantnumberofcasestheacutephaseofrheumatic
fevergoesunnoticed.
• Thispatientwasawhitemanaged38years.Hehadno
previoushistoryofrheumaticfever.
UniversityofCapeTown
Clinicalfindings
• Hepresentedwithswellingoftheabdomenforonemonth.
• Hehadalsonoticedbreathlessnessandpalpitations.On
examinationhewasorthopnoeic.
• Therewasoedemaofthelegsandscrotum.
• Hispulsewasveryrapidandtotallyirregular.
• Theheartwasenlargedwithanearlydiastolicmurmurin
mitralarea.
• Theliverwasgreatlyenlarged.
• Signsofcardiacfailureworsenedandthepatientdiedasa
consequence.
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Pathology
• Across-sectionoftheleftsideoftheheart,viewingtheleft
atriumfromabove.
Aorta
LA
UniversityofCapeTown
• Themitralvalve
curtainsare
adhesed,
thickened,
distortedand
calcified,resulting
infunnelstenosis.
• Theorificeis
narrowedtoa
typical“fishmouth”
or"buttonhole”.
• ".
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PhotobyCindySeigle,CreativeCommonsBY-NC-SA
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• Hereisthetransected
leftventricle,viewing
theabnormalmitral
valvefrombelow.
• Thechordae
tendineaearealso
thickenedand
retracted(shortened)
byfibrosis.
UniversityofCapeTown
TheresultsofchronicRHD
• Theconsequencesofvalvedamageareheartmurmurs,cardiac
hypertrophyand/ordilatationandcardiacfailure.
• Othercomplicationsarearrhythmias,thromboembolicevents
(suchasstroke)andinfectiveendocarditis.
UniversityofCapeTown
Comment
• Notethatour2casesdate
fromthe1920’sand1930’s.
• Atthistimethelinkbetween
streptococcalsorethroat
andrheumaticfeverwas
recognisedbutnotfully
understood.
• Penicillinprophylaxisto
prevent(recurrent)attacksof
rheumaticfeveronlycame
intouseafterWorldWarII.
Imageusedwithpermissionfromthe
DepartmentofMicrobiology,UCT
UniversityofCapeTown
Streptococci
α-haemolytic
β-haemolytic
GroupAStreptococcuspyogenes
rheumaticfever,scarletfever,glomerulonephritis
ImageusedwithpermissionfromtheDepartmentofMicrobiology,UCT
UniversityofCapeTown
• Life-savingvalvereplacementsurgerywasonlydevelopedin
the1960’s.
UCTprostheticmitralvalve
PM239/1968
Starr-Edwardsmitralvalve
prosthesis
PM239/1978
• Currently,approximately150rheumaticvalvesarereplaced
eachyearbythecardiothoracicsurgeryunitatGroote
SchuurHospital.
UniversityofCapeTown
Links&references
• Rheumaticheartdiseaseisstillveryimportantindeveloping
countriessuchasSouthAfrica,andevenincrowded,
economicallydepressedurbanareasintheWesternworld.See
thisWHOmapsummarisingtheepidemiologyhttp://www.who.int/
cardiovascular_diseases/en/cvd_atlas_02_rheumaticHD.pdf
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